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Biomedical subjects

W J Litchy

Publications and source records attributed to W J Litchy.

At least 19 recordsLinked to original sources

Hemimasticatory spasm: clinical and electrophysiologic observations.

Hemimasticatory spasm is a rare disorder of the trigeminal nerve that produces involuntary jaw closure due to paroxysmal unilateral contraction of jaw-closing muscles. We report three patients with this disorder. Electrophysiologic studies demonstrated normal blink and masseter reflexes. The masseter inhibitory reflex was absent during periods of spasm. Needle electromyography demonstrated irregular bursts of motor unit potentials that were identical to the pattern observed in hemifacial spasm. The electrophysiologic findings suggest ectopic excitation of the trigeminal motor root or its nucleus, an abnormality that is analogous to ectopic excitation of the facial nerve in hemifacial spasm. One patient improved temporarily with surgery, one improved while on treatment with carbamazepine, and another responded favorably to botulinum toxin injection.

Adult

The Rochester Diabetic Neuropathy Study: reassessment of tests and criteria for diagnosis and staged severity.

We evaluated the initial assessments of the 380 diabetic patients with and without polyneuropathy in the Rochester Diabetic Neuropathy Study for (1) associations among neuropathy test results, (2) usefulness of different tests for diagnosing and staging polyneuropathy, (3) appropriateness of different minimal criteria for the diagnosis of polyneuropathy, and (4) significant differences in test results with increasing stage of polyneuropathy. Nerve conduction ([NC]; abnormality in two or more nerves) and quantitative autonomic examination ([QAE]; decreased heart-beat response to deep breathing [DB] or the Valsalva maneuver [VAL]) were the most sensitive and objective and were especially suitable for detection of subclinical neuropathy. We propose the following minimal criteria for the diagnosis of diabetic polyneuropathy: greater than or equal to 2 abnormal evaluations (from among neuropathic symptoms, neuropathic deficits, NC, quantitative sensory examination [QSE], and QAE) with one of the two being abnormality of NC or QAE (DB or VAL). Neuropathy Symptom Score, Neuropathy Disability Score, QSE (vibratory or cooling detection threshold), and summated compound muscle action potential of ulnar, peroneal, and tibial nerves were best for judging severity. Inability to walk on heels provided a discrete separation of diabetic patients into those with mild and those with more severe neuropathy--a separation helpful in staging.

Diabetic Neuropathies

Effect of BAEP monitoring on hearing preservation during acoustic neuroma resection.

We measured the effect of brainstem auditory evoked potential (BAEP) monitoring on hearing preservation in acoustic neuroma resection in 90 consecutive patients with monitoring compared with 90 historical controls matched for tumor size and preoperative hearing status. In small tumors (less than 2 cm), BAEP monitoring was associated with a higher rate of hearing preservation and a greater chance that the hearing preserved was clinically useful. Changes in the BAEP intraoperatively showed a good correlation with postoperative hearing status.

Evoked Potentials, Auditory, Brain Stem

Physiological prediction of muscle forces--I. Theoretical formulation.

A physiological model for predicting muscle forces is described. Rigid-body mechanics and musculoskeletal physiology are used to describe the dynamics of the segment model and muscle model. Unknown muscle and joint contact forces outnumber the equilibrium equations resulting in an indeterminate problem. Mathematical optimization is utilized to resolve the indeterminacy. The modeling procedure relies entirely on established physiological principles. Data describing the muscle anatomy and body structures are included. A model defining the force-length-velocity-activation relationship of a muscle is adopted. The force a muscle produces is assumed to be proportional to its maximum stress, physiological cross-sectional area, activation, and its functional configurations including the muscle architecture, muscle length, contracting velocity, and passive tension. These factors are incorporated into inequality equations which limit the force for each muscle. Minimal muscular activation is forwarded as the optimization criterion for muscle force determination.

Biomechanical Phenomena

Physiological prediction of muscle forces--II. Application to isokinetic exercise.

The successful application of a physiological model of the musculoskeletal system capable of accounting for nonequilibrium dynamic loading and predicting individual muscle forces in the knee is presented. The model incorporates rigid-body mechanics and musculoskeletal physiology. Unknown muscle and joint contact forces outnumber the equilibrium equations resulting in an indeterminant problem. Mathematical optimization is utilized to resolve the indeterminacy. The model is used to estimate individual muscle forces during isokinetic exercise. Five subjects were tested at speeds of 60 degrees/s and 180 degrees/s. A newly proposed optimal criterion, minimizing muscular activation, results in muscle force predictions which have significantly higher correlations with myoelectric activity than other linear and nonlinear optimal criteria. The results demonstrate that properly constrained linear programming methods do not limit the number of active muscles and allow for uniform recruitment of the active muscles.

Adult

Selective peripheral denervation for torticollis: preliminary results.

Herein we report the preliminary results in nine patients who have undergone selective peripheral denervation for spasmodic torticollis and have been followed up for at least 13 months. All patients had improvement immediately after surgical intervention, and the results have been maintained in five patients. In one patient who had recurrent torticollis, a second procedure in conjunction with injection of botulinum toxin has produced substantial improvement; however, follow-up was brief (6 months). No surgical complications occurred. We believe that selective peripheral denervation is safe and that it can benefit patients with torticollis who have not responded to other types of therapy. These favorable results confirm other published reports on the efficacy of selective peripheral denervation. Long-term follow-up, however, is necessary for determining the role of this procedure in the management of torticollis.

Adult

Effects of alterations in pulmonary function and sleep variables on survival in patients with amyotrophic lateral sclerosis.

Breathing abnormalities and nocturnal hypoventilation occur in patients with amyotrophic lateral sclerosis (ALS). A prospective study was undertaken to determine the relationship of pulmonary function test abnormalities with quality of sleep and survival in 21 patients with ALS. Results of spirometry including determination of maximal respiratory pressures and arterial blood gases were compared with several formal polysomnographic variables and then also with 18-month survival. The patients had mild to moderate pulmonary function deficits, but the quality of sleep was best related to age (mean age, 58.5 years). The results of pulmonary function tests and arterial blood gas measurements did not correlate well with the presence of nocturnal breathing events or survival time, but the maximal inspiratory pressure was 86% sensitive for predicting the presence of a nocturnal oxygen saturation nadir of 80% or less and 100% sensitive for predicting 18-month survival. Although obstructive breathing events occurred, the primary explanation for the decline in nocturnal oxygen saturation was hypoventilation. We conclude that routine pulmonary function tests may be useful for screening for reductions in nocturnal oxygen saturation and also may have prognostic value. Further studies may determine whether treatment of nocturnal hypoventilation will have an effect on survival in patients with ALS who have breathing impairment.

Adult

Late effects of paralytic poliomyelitis in Olmsted County, Minnesota.

We identified a cohort of 300 individuals who had paralytic polio between 1935 and 1955. All lived in Olmsted County, Minnesota. From the 247 survivors, we selected 50 subjects for detailed historical, functional, psychological, clinical, and electrophysiologic evaluation. Sixty-four percent of these 50 survivors complained of new symptoms of muscle pain, fatigue, and weakness after a period of prolonged stability. This led to changes in lifestyle or activity in only 18%. The likelihood of expressing new complaints was not related to present age or interval since polio, and electrophysiologic testing did not distinguish between those with or without new problems. The development of new difficulties in a limb was most strongly predicted by significant paralysis of that limb at the time of the acute illness. Patients with leg weakness were twice as likely to complain of new problems compared to those with arm weakness. Elevated creatine kinase levels were present only in those with new complaints.

Cohort Studies

The Rochester Diabetic Neuropathy Study: design, criteria for types of neuropathy, selection bias, and reproducibility of neuropathic tests.

A cross-sectional survey and subsequent longitudinal study among diabetic residents of Rochester, MN--The Rochester Diabetic Neuropathy Study (RDNS)--is population-based and uses quantitative, validated, and unique end points to detect, classify, and stage neuropathy. Nondiabetic persons, drawn from the same population, serve as controls. For patients 10 to 70 years old, the RDNS cohort is representative of diabetics living in Rochester, MN. We assessed reproducibility of tests used to characterize and quantitate severity of neuropathy in 20 diabetic subjects without neuropathy and with varying severities of neuropathy. Using intraclass correlation coefficient (rI) as a measure of test reproducibility, we found high rI (usually 0.9 or better) with small confidence intervals for the Neurologic Disability Score (NDS); weakness subset of NDS (W-NDS); vibratory and cooling detection thresholds (using computer-assisted sensory examination [CASE] IV); compound muscle action potentials; sensory nerve action potentials; and motor nerve conduction velocities. There was good agreement among three trained observers for NDS and the W-NDS.

Action Potentials

Wohlfart-Kugelberg-Welander syndrome: serum creatine kinase and functional outcome.

The medical records of 31 patients (19 male and 12 female) with clinical and electrophysiologic features of Wohlfart-Kugelberg-Welander syndrome were reviewed. The reported age at onset ranged from less than one year to 46 years, and the age at diagnosis ranged from three to 66 years. Proximal muscle weakness, especially of the lower extremities, and muscular atrophy were the predominant clinical features. Elevated serum creatine kinase levels were noted in four female and 12 male patients, and the degree of elevation was higher in the male patients (up to 32 times the upper limit of normal) than in the female patients. On initial evaluation, two patients were wheelchair-bound, whereas the others were ambulatory. On follow-up evaluation three to 32 years later (mean, 15.5 years), 11 patients used wheelchairs, although only three were wheelchair-bound. The disease followed a steady, slowly progressive course. The outcome of ambulatory status did not correlate with the initial creatine kinase determination.

Activities of Daily Living

A comparison of magnetic and electrical stimulation of spinal nerves.

The utility of the magnetic coil for stimulation of the cervical spinal nerves was compared to electrical stimulation by a monopolar needle cathode placed onto the spinal lamina in six volunteers. No statistical difference in average amplitudes or areas of evoked CMAPs was found although the size of the magnetic coil evoked potentials was less at C7-8 in several subjects. Electrical stimulation resulted in depolarization at a more proximal site. Electrical stimulation was associated with more discomfort, especially at C5-6. We conclude that electrical stimulation using a monopolar needle as the cathode is the superior technique for the clinical electrophysiologic study of the proximal brachial plexus and cervical spinal nerves.

Action Potentials

Inclusion body myositis. Observations in 40 patients.

Inclusion body myositis (IBM) was suspected on light microscopic grounds in 48 of 170 consecutive patients with inflammatory myopathies. One or more vacuoles containing membranous material, groups of atrophic fibres, and an autoaggressive endomysial inflammatory exudate occurred in 100, 96 and 92% of the muscle specimens. All three of these features were present in 88% of the specimens. Electron microscopy confirmed the presence of filamentous inclusions in 40 of 43 patients. The inclusions are typically near vacuoles and a minimum of three vacuolated fibres must be scrutinized to detect them with confidence. There is no electromyographic pattern that can reliably distinguish IBM from other inflammatory myopathies. The typical clinical features in the patients diagnosed by histological criteria as IBM were: insidious onset after age 50 yrs with painless, proximal lower extremity weakness; slow but relentless progression with selectively severe involvement of quadriceps, iliopsoas, tibialis anterior, biceps and triceps muscles; relatively early depression of the knee reflexes; and a normal or mildly elevated serum creatine kinase level. The male: female ratio was 3:1. Distal weakness occurred in about 50%, but only in 35% was it as great or greater than proximal weakness. Significant associated illnesses include other autoimmune disorders (15%), diabetes mellitus (20%), and diffuse peripheral neuropathy (18%). Prednisone treatment at dose levels frequently effective in polymyositis failed to prevent disease progression in those patients observed for 2 or more years. Our findings support the notion that IBM is a distinct entity in which a set of pathological features is associated with a constellation of clinical findings.

Adult

Transdiaphragmatic twitch pressure. Effects of lung volume and chest wall shape.

The effect of lung volume and thoracoabdominal shape on the transdiaphragmatic twitch pressure (Pdit) amplitude was evaluated in six volunteers during airway occlusion. Twitch stimulation was applied through fine wire electrodes implanted near both phrenic nerves. Stimulations were tolerated with little discomfort and constant phrenic nerve responses were maintained for hours. At FRC the group mean Pdit was 31.4 cm H2O (range, 19 to 36 cm H2O), and its coefficient of variation ranged between 2 and 5% in individual subjects. At 1 L above FRC, the Pdit decreased a mean of 7.8 cm H2O (range, 2.8 to 11.9 cm H2O). This change was caused primarily by a decrease in esophageal pressure amplitude. The shape of the relaxed chest wall was altered by loading the rib cage with a force of 5 to 9 kg. Load and shape had little effect on Pdit independently of lung volume. Our modified technique of phrenic nerve stimulation through small wire electrodes is ideally suited for longitudinal intervention studies in patients. We conclude that the variability of Pdit with shape is small compared with its expected decrease with lung volume.

Action Potentials

Trochanteric sciatic neuropathy.

A patient with severe weakness, atrophy, and sensory loss of the right leg had a focal right sciatic neuropathy. The sciatic nerve was enlarged at the level of the lesser trochanter, excessively firm, and multistranded; its stimulation threshold was focally increased. Biopsied fascicles had reduplicated perineurial leaflets, many Renaut bodies, and an abnormal unimodal spectrum of small-diameter fibers. We postulate that the lesion was induced by the combination of an underlying prominent lesser trochanter and sitting on hard benches.

Atrophy

Distinction between neoplastic and radiation-induced brachial plexopathy, with emphasis on the role of EMG.

The results of clinical, radiologic, and electrophysiologic studies are retrospectively reviewed for 55 patients with neoplastic and 35 patients with radiation-induced brachial plexopathy. The presence or absence of pain as the presenting symptom, temporal profile of the illness, presence of a discrete mass on CT of the plexus, and presence of myokymic discharges on EMG contributed significantly to the prediction of the underlying cause of the brachial plexopathy. The distribution of weakness and the results of nerve conduction studies were of no help in distinguishing neoplastic from radiation-induced brachial plexopathy.

Adult

Lumbar radiculopathy after spinal fusion for scoliosis.

In 184 patients with no preoperative neurologic deficit who underwent operation for idiopathic scoliosis, somatosensory evoked potential monitoring was used. Four patients had neurologic deficits postoperatively. Two patients developed mild signs of intraspinal lesions involving upper motor neurons at high lumbar levels that resolved over 3-5 months. These patients and two others developed evidence of unilateral, moderate, lower motor neuron damage that was confirmed on electromyography. No changes in somatosensory evoked potentials occurred in these patients. Lumbar root damage may be difficult to recognize after operation and should be considered in patients with neurologic deficit after scoliosis surgery.

Adolescent

The utility of magnetic stimulation for routine peripheral nerve conduction studies.

The magnetic stimulator was compared with percutaneous electrical stimulation for activation of the median nerve at the wrist in four volunteers. Magnetic stimulation was unable to achieve supramaximal stimulation without activation of the ulnar nerve. The point of nerve depolarization could not be reliably estimated from the relationship of the stimulator head to the nerve. We conclude that the magnetic stimulator coil is as yet not suitable for routine electrodiagnostic use in the peripheral nervous system.

Action Potentials